Wegener's granulomatosis associated with antiphospholipid syndrome

Lupus. 2000;9(9):717-20. doi: 10.1191/096120300675018039.


We report a case of Wegener's granulomatosis (WG) with pulmonary hemorrhage also satisfying the criteria for antiphospholipid antibody syndrome (APS). This association has, to the best of our knowledge, never been described before. Pulmonary hemorrhage may be an early manifestation of several immune and idiopathic disorders such as ANCA-associated vasculitis. Several case-reports of APS patients with capillaritis have been described. A possible explanation is that microvascular thrombosis with subsequent increase in vascular permeability facilitates perivascular IgG and complement deposition leading to development of capillaritis. Whether the vascular disease is secondary to thrombosis or vasculitis or both is important in choosing the proper management strategy. We suggest that anticardiolipin antibodies (aCL) should be detected in ANCA-associated vascularitis because they may contribute to life-threatening events superimposed on vascular damage.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anti-Inflammatory Agents / therapeutic use
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / complications*
  • Antiphospholipid Syndrome / drug therapy
  • Antiphospholipid Syndrome / physiopathology
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Granulomatosis with Polyangiitis / complications*
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / physiopathology
  • Hemorrhage / etiology
  • Hemorrhage / physiopathology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Insulin / therapeutic use
  • Lung Diseases / etiology
  • Lung Diseases / physiopathology
  • Male
  • Prednisone / therapeutic use
  • Tomography, X-Ray Computed
  • Warfarin


  • Anti-Inflammatory Agents
  • Antibodies, Antineutrophil Cytoplasmic
  • Anticoagulants
  • Immunosuppressive Agents
  • Insulin
  • Warfarin
  • Cyclophosphamide
  • Prednisone